Paracentral Acute Middle Maculopathy May Be Only Sign of Giant Cell Arteritis

Patients with giant cell arteritis (GCA) often present with isolated findings of paracentral acute middle maculopathy (PAMM), according to findings published in Retina.

Patient records collected between 2016 and 2020 at the Rothschild Foundation Hospital in France were retrospectively reviewed. All patients (N=96) with a diagnosis of GCA were assessed for ophthalmic features and by optical coherence tomography (OCT). Patients were stratified by PAMM, central retinal artery occlusion (CRAO), and anterior ischemic optic neuropathy (AION) status. The investigators also discussed 4 specific cases.

The study reviewed 96 patients (mean age 75.6±8.0 years, 58 women). Eyes were stratified into cohort 1 (16.7%; isolated PAMM or in combination with CRAO or AION), cohort 2 (37.5%; CRAO or AION without PAMM), or cohort 3 (45.8%; no AION, CRAO, or PAMM).

Cohorts 1 and 2 did not differ by symptoms of GCA, baseline visual acuity, biological markers, or medical history. Patients with PAMM tended to be 5 years older than patients without PAMM (P =.020) and PAMM associated with higher visual acuity with both middle and inner retinal infarction (P =.0265).

Patients in cohort 3, who did not have ophthalmologic involvement, were significantly younger than patients with ophthalmologic involvement (cohorts 1 and 2; P =.0002), they had lower blood pressure (P =.0085), higher hemoglobin (P =.0466), and fewer had jaw claudication (P <.0001).

Patient 1 was a 70-year-old who presented at the emergency department with bilateral vision loss, jaw claudication, and hypersensitivity of the scalp. OCT found a fern-like PAMM pattern and choroidal ischemia in the inferior papillary and macular areas. GCA was diagnosed by temporal artery biopsy. After 3 days of steroid treatment, the patient had transient visual loss in the right eye and was switched to iloprost. At 1-year the visual acuity was 20/25 in the right eye.

Patient 2 was an 84-year-old with sudden bilateral visual loss. Fundus examination found bilateral optic disc edema and OCT identified diffuse PAMM. GCA was diagnosed due to bilateral AION, elevated C-reactive protein levels, and ophthalmic features observed during magnetic resonance imaging. The patient was given intravenous steroids. At 1 year the visual acuity remained stable at 20/4000 in the right eye and 20/20 in the left.

Patient 3 was an 86-year-old who presented at the emergency department with decreased visual acuity of the left eye. The patient had lower parapapillary hemorrhage compatible with AION and OCT identified parafoveolar temporal diffuse PAMM. GCA was confirmed by biopsy of the temporal artery. After corticosteroid treatment, the patient did not have improved vision.

Patient 4 was an 81-year-old with decreased right vision, jaw claudication, and shoulder pain. A fundus examination found a white ischemic area and optic disc swelling. OCT showed PAMM lesions, diffuse middle and inner retinal ischemia, and cotton wool spots in the cilioretinal artery territory. Steroid therapy improved visual acuity to 20/50 in the right eye.

These data indicated that older adults with GCA may present with PAMM and elderly patients with PAMM should be assessed for GCA.

Reference

Mairot K, Sené T, Lecler A, et al. Paracentral acute middle maculopathy in giant cell arteritis. Retina. Published online October 25, 2021. doi:10.1097/IAE.0000000000003339